Cerebral Palsy

Ahmed Abdelmoity, M.D, FAAP

Associate professor of Pediatric Neurology
American Board Certified in Pediatrics
American Board Certified in Neurology
American Board certified in Clinical Neurophysiology
Director of the comprehensive epilepsy program
Director of the Clincal Neurophysiology
University of Missouri at Kansas city
Children’s Mercy Hospitals
Neuroanatomy and physiology
background
What is CP
 The term was originally coined more than a
century ago. Meaning “brain paralysis”
 Static encephalopathy, with brain cell
damage, leading to different disabilities
 CP is permanent from a structural stand point
leading to a group of disorders affecting
development, posture, and movement.
What is CP
 Age of onset: From fetal period to 3 years
 Lesions after 3 years may manifest like CP
but by definetion not CP
 Lesion location: Brain ONLY. diseases
specific to the peripheral nerves or spinal cord
(eg, spinal muscular atrophy,
myelomeningocele) or to the muscles (eg,
muscular dystrophies), although causing early
motor abnormalities, are not considered CP.
Associated findings
 Mental retardation in 30-50%
 Epilepsy in 15-60% of children
 Respiratory complications
 Visual problems
 Retinopathy of prematurity
 Cortical blindness
 Nutrition
 Failure to thrive
 Swallowing problems.
Types of CP
 Spastic hemiplegia - affecting one side of the body, with upper extremity
spasticity more than lower extremity spasticity
 Spastic diplegia - affecting lower extremities more than upper extremities
 Spastic quadriplegia - CP affecting all 4 extremities (full body)
 Dyskinetic CP (athetoid CP, choreoathetoid CP, and dystonic CP) - CP
with extrapyramidal signs with abnormal movements
 Mixed CP - CP with no single specific tonal quality predominating;
typically characterized by a mixture of spastic and dyskinetic components
 Hypotonic CP - CP with truncal and extremity hypotonia with
hyperreflexia and persistent primitive reflexes;
Pathophysiology
 Result from underlying structural abnormality
of the brain.
 Early prenatal, perinatal or postnatal injury
 Due to Vasculr insufficiency, tonins,
infection, prematurity
 Prenatal factors result in 70-80% of CP cases
 In most, the exact cause is unknown
Brain development
 Primary neurulation - Weeks 3-4 of gestation
 Prosencephalic development - Months 2-3 of
gestation
 Neuronal proliferation - Months 3-4 of gestation
 Neuronal migration - Months 3-5 of gestation
 Organization - Month 5 of gestation to years
postnatal
 Myelination - Birth to years postnatal
 Pathophysiology Cont’d
 Injury before gestational age:
 20th week: neuronal migration deficit
 Between 26th and 34th weeks can result in
periventricular leukomalacia;
 Injury between the 34th and 40th weeks can result in
focal or multifocal cerebral injury.
 Injury due to vascular insufficiency depends on
 Vascular distribution to the brain
 Compensatory mechanisms.
Epidemiology
 In developed countries: 2-2.5 per 1000 live
births
 Prevalence is much higher is very preterms
 In developing countries, prevalence is not
well established. Estimated 1.5-5.6 per 1000
live births.
 Lower socioeconomic class is higher risk
 Males “may be at higher risk” (Stanley 2000)
Pathophysiology Cont’d
 The periventricular area is hypoperfused in early pregnancy
 26-34 weeks, It is very susceptible to injury
 Grade I - This is subependymal and/or germinal matrix
hemorrhage.
 Grade II - This is subependymal hemorrhage with extension
into the lateral ventricles without ventricular enlargement.
 Grade III - This is subependymal hemorrhage with extension
into the lateral ventricles with ventricular enlargement.
 Grade IV - A germinal matrix hemorrhage that dissects and
extends into the adjacent brain parenchyma
Diagnosis
 History:
 Prenatal history
 Perinatal history
 Developmental history
 Need for adaptive equipment
 General medical history
 Physical
Prenatal history
 Exposure to drugs, toxins, or alcohol
 Co-morbid medical conditions such as
maternal diabetes, acute mental illness,
radiation exposure..etc.
 Trauma, prenatal care, fetal movements
 History of previous abortions, Parental
consanguinity, and family history to assess
for genetic disorders
Perinatal history
 Gestational age (prematurity is a risk for CP)
 Delivery type and presentation. Vaginal
malpresentation is a risk for CP
 Birth weight, APGAR score, and neonatal
complications
 Early neonatal responses: neonatal reflexes,
feeding, irritability, and level of
consciousness.
Developmental history
 Gross motor, fine motor, language, and social
milestones after birth
 Head control by 3 months, roll at 4 months, sit at 6
months, walk by 15 months
 The presence of unexplained regression would be
more suggestive of genetic rather than CP
 Current social skills, academic performance
 Standardized tests would be helpful for full
assessment
Need for adaptive equipment
 Review patient’s need for equipment such as:
 Adaptive devices eg: computer assisted speech
program
 Orthotics eg; ankle-foot orthoses, walkers,
wheelchairs…etc.
 Need for home modifications: eg bath rail, toilet
elevations, special mattress.
General medical history
 Swallowing and feeding
 Respiration, and chest condition
 Digestion. (constipation or distention…etc.)
 Musculoskeletal: Pain, deformity, growth
 Nutrition and risk for failure to thrive
 Vision, and need for glasses
Physical exam
 Initially hypotonia due to “shock state”
 Spasticity follows (6-12 months of life)
 Abnormal neck and trunk tone
 Asymmetric posture, strength coordination or
gait
 Increased reflexes
 Persistence of primitive reflexes such as Moro,
and asymmetric tonic nex reflex
Physical exam
 Spasticity: velocity dependent increased tone
 Antigravity muscles more involved: Elbows flexed,
and legs extended
 Extrapyramidal CP: may have axial hypotonia, and
motor dysfunction
 Athetosis (ie, slow, writhing, involuntary movements, particularly in the distal extremities)
 Chorea: (ie, abrupt, irregular, jerky movements)
 Dystonia (ie, slow rhythmic movements with increased muscle tone and abnormal postures,
eg, in the jaw and upper extremities)
Classic physical presentations
of different types of CP
Spastic hemiplegic CP

 One-sided upper motor neuron deficit

 Arm generally affected more than leg; possible early hand
preference or relative weakness on one side; gait possibly
characterized by circumduction of lower extremity on
affected side
 Specific learning disabilities
 Oromotor dysfunction
 Possible unilateral sensory deficits
 Visual-field deficits (eg, homonymous hemianopsia) and
strabismus
 Seizures
Spastic diplegic CP

 Upper motor neuron findings in the legs more

than the arms
 Scissoring gait pattern with hips flexed and
adducted, knees flexed with valgus, and
ankles in equinus, resulting in toe walking
 Learning disabilities and seizures less
commonly than in spastic hemiplegia
Spastic quadriplegic CP
 All limbs affected, either full-body hypertonia or
truncal hypotonia with extremity hypertonia
 Oromotor dysfunction
 Increased risk of cognitive difficulties
 Multiple medical complications (see Complications)
 Seizures
 Legs generally affected equally or more than arms
 Categorized as double hemiplegic if arms more
involved than legs
Dyskinetic (extrapyramidal) CP

 Early hypotonia with movement disorder emerging

at age 1-3 years
 Arms more affected than legs
 Deep tendon reflexes usually normal to slightly
increased
 Some spasticity
 Oromotor dysfunction
 Gait difficulties
 Truncal instability
 Risk of deafness in those affected by kernicterus
Etiology
 May be multifactorial
 In most cases is unknown
Maternal and prenatal risk factors
 Long menstrual cycle
 Previous pregnancy loss
 Previous loss of newborn
 Maternal mental retardation
 Maternal thyroid disorder, especially iodine deficiency
 Maternal seizure disorder
 History of delivering a child weighing less than 2000 g
 History of delivering a child with a motor deficit, mental
retardation, or a sensory deficit
Factors during pregnancy
 Polyhydramnios
 Treatment of the mother with thyroid hormone
 Treatment of the mother with estrogen or progesterone
 Maternal seizure disorder
 Maternal severe proteinuria or high blood pressure
 Maternal methyl mercury exposure
 Congenital malformations in the fetus
 Male sex of fetus
 Bleeding in third trimester
 Intrauterine growth retardation
 Multiple gestation: may relate more to prematurity or intrauterine growth
retardation.
Perinatal factors
 Prematurity
 Chorioamnionitis
 Nonvertex and face presentation of the fetus
 Birth asphyxia
Birth Asphyxia
 In 10% or less of cases, birth asphyxia can be determined as
the definitive cause.
 Even when birth asphyxia is thought to be cause, abnormal
prenatal factors (eg, intrauterine growth retardation,
congenital brain malformations) could have been the cause
 Cases of CP attributed to birth asphyxia must document:
 Clear evidence of acidosis,
 Moderate-to-severe neonatal encephalopathy
 Restriction to spastic quadriplegia, dyskinetic or mixed types of CP
 Exclusion of other etiologies.
Birth Asphyxia Cont’d
 Intrapartum event must be suggested by:
 a sentinel event,
 fetal heart rate changes,
 Apgar score less than 4 at 5 minutes,
 organ system damage related to tissue hypoxia,
and
 early imaging abnormalities
 Low apgar scores can be due to other causes
such as infection.
Post natal factors
 Infections (eg, meningitis, encephalitis)
 Intracranial hemorrhage (eg, due to prematurity,
vascular malformations, or trauma)
 Periventricular leukomalacia (in premature infants)
 Hypoxia-ischemia (eg, from meconium aspiration)
 Persistent fetal circulation or persistent pulmonary
hypertension of the newborn
 Kernicterus
Possible causes by type
Spastic hemiplegic
 70-90% are congenital and 10-30% are acquired (eg,
vascular, inflammatory, traumatic).
 In unilateral lesions, the middle cerebral artery on
the left is involved twice as commonly as the right.
 Other structural brain abnormalities include
hemibrain atrophy and posthemorrhagic
porencephaly.
 In premature infants, this may result from
asymmetric periventricular leukomalacia.
Spastic diplegic
 In premature infants, spastic diplegia may
result from parenchymal-intraventricular
hemorrhage or periventricular leukomalacia.
 In term infants, no risk factors may be
identifiable or the etiology might be
multifactorial.
Spastic quadriplegic
 Approximately 50% are prenatal, 30% are perinatal, and 20%
are postnatal in origin.
 Associated with cavities that communicate with the lateral
ventricles, multiple cystic lesions in the white matter, diffuse
cortical atrophy, and hydrocephalus.
 Often history of difficult delivery with evidence of perinatal
asphyxia.
 Preterm infants may have periventricular leukomalacia.
 Full-term infants may have structural brain abnormalities or
cerebral hypoperfusion in a watershed (ie, major cerebral
artery end zone) distribution.
Dyskinetic (extrapyramidal)
 This type may be associated with
hyperbilirubinemia in term infants or with
prematurity without prominent
hyperbilirubinemia.
 Hypoxia affecting the basal ganglia and
thalamus may affect term infants more than
preterm infants.
Workup
 The 2004 AAN practice parameter suggests
testing for coagulation if a cerebral infarction
is seen. However, available data were
insufficient.
 The 2004 AAN practice parameter did not
recommend an EEG unless suspicion for
epilepsy.
Labwork
 Lactate and pyruvate values: may indicate a mitochondreal disorder
 Thyroid function studies: may be related to abnormalities in muscle tone
or deep tendon reflexes or to movement disorders.
 Ammonia level: If elevated may indicate liver or urea cycle dysfunction.
 Serum quantitative amino acid and urine quantitative organic acid values:
These studies may reveal inherited metabolic disorders.
 Chromosomal analysis, including karyotype analysis and specific DNA
testing: These may be indicated to rule out a genetic syndrome, if
dysmorphic features or abnormalities of various organ systems are present.
Neuro-imaging
 Head US: In unstable infants
 Head CT: Best to detect bleeding
 MRI: The gold standard for CP patients
 AAN 2004 practice parameter recommends neuroimaging "to
establish that a brain abnormality exists in children with CP,
that may, in turn, suggest an etiology and prognosis."
 MRI is preferred over CT scanning.
 Although the precise role for MRI in the diagnosis and workup of
children with CP or suspected CP has not been fully elucidated,
recent literature suggests that MRI should be strongly considered in
all cases; in one study, 89% children with CP were found to have
abnormal MRIs (Bax, 2006).
 Additionally, MRI may have a role in predicting neurodevelopmental
outcomes in preterm infants (Woodward, 2006).
Other tests
 EEG: only if the patient has seizures or
suspected seizures
 EMG/NCS: Only if the patient has symptoms
to suggest a lower motor neuron involvement
Management
 The 2004 AAN practice parameter suggests screening for the
following potential CP-associated deficits at the initial
assessment:
 Mental retardation
 Ophthalmologic and hearing impairments
 Speech and language disorders
 Oromotor dysfunction
 Various medications may improve spasticity. eg: baclofen,
Tizanidine, benzodiazepines…etc
 Numerous medications, may relieve the movement
difficulties associated with CP. These drugs target dystonia,
myoclonus, chorea, athetosis, and spasticity.
Management
 While antiparkinsonian drugs (eg, anticholinergic and
dopaminergic drugs) and antispasticity agents (eg, baclofen)
have primarily been used in the management of dystonia,
anticonvulsants, antidopaminergic drugs, and antidepressants
have also been tried.
 Anticonvulsants (including benzodiazepines such as
diazepam, valproic acid, and barbiturates) have been useful in
the management of myoclonus. Chorea and athetosis
 Neurologists and rehabilitation medicine (physiatrists) play
significant roles in the management of antispasticity
medications, and PT.
 Seizure disorders are common with CP, and a neurologist
should follow
Management
 Others:
 Melatonin
 Laxatives
 Pulmonary care
 GI care
 Weight control
 Multivitamines
Thank you
Questions